Rhinoscleroma Revisited

ABSTRACT Rhinoscleroma (more appropriately 'scleroma') is a chronic, disfiguring inflammatory lesion that is rarely encountered in the present-day otolaryngology practice. The diagnosis often becomes difficult especially when it recurs and presents late with complications. This report illustrates the clinical and histologic features of rhinoscleroma in a defaulter patient who represents advanced-stage disease with orbital and intracranial extensions. Special emphasis has been provided on the computed tomography impressions such that the evolution of scleroma with time can be studied.

Cytohistological features of rhinoscleroma.

Rhinoscleroma, also known as Mikulicz disease, is a chronic progressive disease caused by Klebsiella rhinoscleromatis. The disease is uncommon in Delhi. There are no cytological references of this disease. We describe the cytological features which should alert the pathologist to rule out this entity, which needs to be treated early. Immunohistochemistry on histopathological material is a useful adjunct to the diagnosis.

Infección por Klebsiella rinoescleromatis: revisión de una entidadotorrinolaringológica de importancia creciente: [revisión] / Klebsiella rhinoscleromatis infection: review of an otolaryngologic entity of increasing relevance: [review]

La infección por Klebsiella rinoescleromatis es una condición infrecuente en nuestro país, pero endémica en ciertas regiones del mundo. Se presenta como una entidad crónica, progresiva y de aspecto granulomatoso. Causa típicamente rinoescleroma, pero puede comprometer otras partes del sistema respiratorio. Especial atención requiere el compromiso laríngeo y traqueal pues puede representar un riesgo para la vida del paciente. Su diagnóstico se basa en hallazgos como deformidad nasal u obstrucción de la vía aérea con disnea, disfonía y estridor respiratorio. Se apoya en técnicas de imagen como la tomografía computada y la resonancia magnética, pero el diagnóstico confirmatorio lo entrega la histopatología. Su tratamiento incluye el uso de antibióticos y cirugía, la que se puede realizar por diversas técnicas. En suma, es una enfermedad que requiere un alto grado de sospecha y se debe tener en consideración actualmente ante la inmigración a nuestro medio desde regiones endémicas cercanas.

Klebsiella Rhinoscleromatis infection is a rare condition in our country, but is endemic in certain regions of the world. It is presented as a chronic entity, progressive and granulomatous aspect.Typically cause rhinoscleroma, but may also compromise other parts of the respiratory tract. Special attention requires the laryngeal and tracheal compromise therefore may pose a risk to the patient’s life. Its diagnosis is based on the clinical findings like nasal deformity or obstruction of the airway with dyspnea, dysphonia and stridor. It relies on imaging techniques such as computed tomography and magnetic resonance imaging, but the diagnosis is confirmed by histopathology. Its treatment includes antibiotics and surgery, which can be achieved by various techniques. In short, is a disease that requires a high degree of suspicion and should be taken into consideration now before the immigration to our country from nearly endemic regions.

Nasal Rhinoscleroma in a Nonendemic Area: A Case Report

Rhinoscleroma is a chronic, granulomatous infectious lesion most frequently affecting the respiratory tract mucosa. This disease is endemic to Africa, Central and South America, South Central and Eastern Europe, the Middle East, and China. We report an extremely rare disease of nasal rhinoscleroma in Korea. The patient was a 63-yr-old man, who suffered from chronic nasal obstruction and septal deviation. Characteristic histology from a nasal biopsy was proven and special stains for bacteria were employed: periodic acid-Schiff, Warthin-Starry silver, Giemsa, and Gram stains. Isolation of Klebsiella rhinoscleromatis from a culture of nasal discharge was failed, but ultrastructural examination of numerous phagocyzed bacilli in the Mikulicz cells confirmed the diagnosis. The histiocytic nature of the Mikulicz cells was confirmed, using CD 68 and alpha-1 antitrypsin, and by the ultrastructural features of Mikulicz cells. Here, we emphasize the recognition of this rare entity in nonendemic regions, frequently leading to delayed diagnosis.

Rinoescleroma / Rhinoscleroma

Presentamos dos observaciones de rinoescleroma (R) en pacientes de sexo femenino de procedencia rural, de 22 y 67 años respectivamente. La primera con lesiones de carácter inflamatorio-proliferativo y la segunda con manifestaciones seudotumorales, localizadas en vías respiratorias altas. El examen microscópico reveló atrofia epitelial, tejido de granulación, infiltrado inflamatorio de tipo crónico a base de linfocitos, plasmocitos, cuerpos de Russell, células de Mikulicz, con bacilos de Von Frisch y fibrosis. No se pudo determinar el período inicial de la enfermedad en ninguno de los casos; lo evidente es que ambas insistieron en que sus lesiones iniciales tenían caracteres de una pápula con erosión en vestíbulos nasales, con propagación hasta los bronquios, sobre todo en la segunda. El tratamiento se hizo con antibióticos de amplio espectro con resultados espectaculares en la primera y con gran mejoría en la segunda. Ambas se encuentran en control

Paranasal sinuses involvement in rhinoscleroma, CT, endoscopic and histopathological study

Rhinoscleroma is a chronic granulomatous inflammatory disease of the upper respiratory tract of an insidous onset, slowly progressive course and benign in nature. This Study was conducted at Oto-Rhino-laryngology and Radiodiagnosis departments in Ain Shams University Hospitals. It included 50 rhinoscleroma patients, 28 females, 22 males, and their age ranged from 17-50 years. All patients were examined by CT-scans of paranasal sinuses, sinoscopy, and histopathological study of suspected tissues. Positive CT. findings were detected in 30 out of 50 cases [60%], while positive maxillary sinus lesions were seen by sinoscopy in 32 out of 50 [64%] in the form of thick polypoidal mucosa, granuloma and atrophic mucosa with secretions, while actual antroscleroma by the histopathological study was found in 11 cases [22%]. Anterior ethmoiditis with occlusion of osteomeatal complex was detected in 16 cases, while anterior and posterior ethmoiditis in 4 cases, sphenoid sinusitis was also seen in 3 cases by CT-scans. In this study, it was found, that CT-scan is reliable and helpfull diagnostic procedure in the detection of extent of rhinoscleroma in paranasal sinuses. Sinoscopy of the paranasal sinuses provided accurate information about the nature of mucosal changes, the presence of secretions and the state of natural ostium

Rinoescleroma / Rhinoescleroma

Se presentan 59 casos de rinoescleroma. Se comenta la etiopatogenia y la patologia de la enfermedad. Se analizan sus factores inmunologicos y se actualiza el tratamiento